Objective　To investigate the operation treatment of gallbladder carcinoma(GBC). Methods The clinical data of 132 patients with GBC from 1980 to 1999 were analysed. Results　The mean age was 55 years and the occurrence sex ratio in female/male was 1.5∶1. Gallstone was found in 80% of the patients, and 87.1% of the GBC were adenocarcinoma. Before December 1990(forward group), simple cholecystectomy were performed on 11 cases, radical cholecystectomy on 9 cases, extended radical cholecystectomy on 5 cases, palliative extra- or intra- drainge on 15 cases, surgical biopsy on 30% cases. Hospital death occurred in 4 cases, the postoperative complications occurred in 20 cases including biliary fistula in 4 cases, pancreatic fistula in 2 cases. Since January 1991(latter group), radical simple cholecystectomy were performed on 2 cases, radical cholecystectomy on 16 cases, extended radical cholecystectomy on 24 cases; palliative extra- or intra- drainge in 9 cases, surgical biopsy on 8 cases. Hospital death occurred in 1 cases; postoperative complications in 12 cases, including biliary fistula in 1 cases, pancreatic fistula in 1 cases. Radical resection rate was 35.7% in forword group, 72.6% in latter group. Conclusions　Surgical tech-nique progress and application with Peng's Multifunctional Operative Dissector can improve the resection rate of GBC. Extended redical resection is an effective and active method to treat middle or late stage GBC.

Phosphodiesterase-4 ( PDE4 ) has been one of the most popular drug targets during recent years due to its critical role in the control of intracellular cyclic adenosine monophos-phate ( cAMP ) concentration and downstream signal transduc-tion. PDE4 is widely distributed in the central nervous system ( CNS) with different expression levels of its four subtypes. Re-cent data indicate that altered PDE4 expression and/or activity is relevant to multiple CNS disorders, such as depression, memory deficiency, drug dependence, and neural lesion. Selective PDE4 inhibitors exhibit therapeutic effects on these disorders and might be a promising pharmacotherapy. The paper highlights recent re-search progress in the roles of PDE4 in CNS function, and dis-cusses the prospects of PDE4 as a novel therapeutic target for CNS disorders.

Objective: This article reported the clinical characteristics and gene mutations of two pseudoachondroplasia cases, and made a literature review in order to improve clinicians′ understanding of the disease. Methods: Clinical features of two patients who were short stature accompanied with skeletal deformities were summarized, and they accepted whole exome sequencing. We also reviewed literature to summarize the clinical characteristics and known gene research progress of all reported Chinese pseudoachondroplasia cases. Results: The two patients′ clinical characteristics were short limbdwarfism with skeletal deformity. Genetic results showed that there were two heterozygous mutations in the cartilage oligomeric matrix protein (COMP) gene of the two patients, c. 1417_1419delGAC and c. 1552G>A, respectively. Up to March 2019, a total of 58 cases of pseudoachondroplasia have been reported in China. The median height of these patients is -5.03 SDS. The clinical features include abnormal gait, short limbs, short fingers/toes, scoliosis, bracelet sign, ankle sign and other skeletal deformities. COMP is the pathogenic gene and mutations mainly located in calmodulin-like domains. The hotspot mutation is c. 1417_1419delGAC. Conclusions Pseudoachondroplasia is a kind of rare genetic disease characterized by short stature and skeletal deformities. The clinical and genetic characteristics of the disease were summarized, which may improve the early diagnosis rate.

Objective:To report the clinical characteristics, diagnosis, and treatment of 2 cases of X-linked acrogigantism(X-LAG).Methods:The clinical information of two patients were retrospectively reported, and peripheral blood DNA was collected for copy number variations detection.Results:Both patients had onset at age of two, with common clinical characteristics including linear growth acceleration, mild facial coarsening, enlargement of hands and feet, increased appetite, and snoring, etc. The heights Z scores of the two patients before treatment were + 6.86 and + 6.53, respectively. Growth hormone(GH) glucose inhibition test showed that GH nadir values were over 1 ng/mL and insulin-like growth factor-Ⅰ(IGF-Ⅰ) were 586.0 ng/mL and 1 042.0 ng/mL, respectively. Patient 1 received three cycles of octreotide microspheres therapy followed by surgery, and achieved clinical and biochemical remission. Patient 2 had lanreotide for 5.5 years but failed biochemical remission. Microduplication of Xq26.3, which contained pathogenic gene G-protein coupled receptor 101(GPR101), was found in germline DNA of two patients through copy number variation detection, leading to the diagnosis of X-LAG.Conclusion:It should be cautious of X-LAG when children below 2 years old presents symptoms such as overgrowth and so on. Medication combined with surgery is effective.